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Total anomalous pulmonary venous connection

Authors
Brian Soriano, MD
David R Fulton, MD
Section Editor
John K Triedman, MD
Deputy Editor
Carrie Armsby, MD, MPH

INTRODUCTION

Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation.

The anatomic variants, clinical manifestations, diagnosis, and management of TAPVC will be reviewed here.

EPIDEMIOLOGY

The incidence of total anomalous pulmonary venous connection (TAPVC) ranges from 0.6 to 1.2 per 10,000 live births [1,2]. Among patients born with congenital heart disease (CHD), the incidence of TAPVC ranges between 0.7 and 1.5 percent. It is the fifth most common cause of cyanotic CHD [1].

EMBRYOLOGY

In normal development, the lung buds are formed from the primitive foregut and share a common vascular plexus (splanchnic plexus), which initially drains into the common cardinal and umbilicovitelline venous systems. With the formation of the lungs (27 to 29 days of gestation), a portion of the splanchnic plexus differentiates into the primitive pulmonary vascular bed [3]. During this same time period, the primitive left atrium forms a primordial evagination (common pulmonary vein) that grows into and joins the pulmonary portion of the splanchnic plexus. Once the connection is made, the primitive pulmonary venous system separates from the cardinal and umbilicovitelline veins. Portions of the common pulmonary vein are subsequently incorporated into the wall of the left atrium and become the two right and two left pulmonary veins, each of which enters the left atrium through a separate orifice.

Total anomalous pulmonary venous connection (TAPVC) arises from the failure of the left atrium to link to the pulmonary venous plexus, which results in the retention of connections to the primitive cardinal and umbilicovitelline drainage systems. The anatomic variants of TAPVC are dependent upon which connections are retained. The cardinal venous system provides connections to the innominate vein, right atrium, superior vena cava, or azygous vein, and the umbilicovitelline system to the portal or hepatic vein, or inferior vena cava.

                       

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Literature review current through: Nov 2016. | This topic last updated: Wed Sep 09 00:00:00 GMT 2015.
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References
Top
  1. Reller MD, Strickland MJ, Riehle-Colarusso T, et al. Prevalence of congenital heart defects in metropolitan Atlanta, 1998-2005. J Pediatr 2008; 153:807.
  2. Hoffman JI, Kaplan S. The incidence of congenital heart disease. J Am Coll Cardiol 2002; 39:1890.
  3. NEILL CA. Development of the pulmonary veins; with reference to the embryology of anomalies of pulmonary venous return. Pediatrics 1956; 18:880.
  4. Keane JF, Fyler DC. Total anomalous pulmonary venous return. In: Nadas' Pediatric Cardiology, 2nd, Keane JF, Lock JE, Fyler DC (Eds), Saunders Elsevier, Philadelphia 2006. p.773.
  5. Duff DF, Nihill MR, McNamara DG. Infradiaphragmatic total anomalous pulmonary venous return. Review of clinical and pathological findings and results of operation in 28 cases. Br Heart J 1977; 39:619.
  6. Seale AN, Uemura H, Sethia B, et al. Total anomalous pulmonary venous connection to the supradiaphragmatic inferior vena cava. Ann Thorac Surg 2008; 85:1089.
  7. Kirshborn P, Jaggers J, Underleider R. Total anomalous pulmonary venous connection. In: Pediatric Cardiac Surgery, 3rd ed, Mavroudis C (Ed), Mosby, Philadelphia 2003. p.612.
  8. Seale AN, Uemura H, Webber SA, et al. Total anomalous pulmonary venous connection: morphology and outcome from an international population-based study. Circulation 2010; 122:2718.
  9. Geva T, van Praagh S. Anomalies of the pulmonary veins. In: Moss and Adams' heart disease in infants, children, and adolescents: including the fetus and young adult, 7th, Allen HD, Shaddy RE, Driscoll DJ, Feltes TF (Eds), Lippincott Williams & Wilkins, Philadelphia 2008. p.761.
  10. Gathman GE, Nadas AS. Total anomalous pulmonary venous connection: clinical and physiologic observations of 75 pediatric patients. Circulation 1970; 42:143.
  11. van Praagh S. Cardiac malpositions and the heterotzxy syndromes. In: Nadas' Pediatric Cardiology, 2nd, Keane JF, Lock JE, Fyler DC (Eds), Saunders Elsevier, Philadelphia 2006. p.675.
  12. van der Velde ME, Parness IA, Colan SD, et al. Two-dimensional echocardiography in the pre- and postoperative management of totally anomalous pulmonary venous connection. J Am Coll Cardiol 1991; 18:1746.
  13. Kim TH, Kim YM, Suh CH, et al. Helical CT angiography and three-dimensional reconstruction of total anomalous pulmonary venous connections in neonates and infants. AJR Am J Roentgenol 2000; 175:1381.
  14. Choe YH, Lee HJ, Kim HS, et al. MRI of total anomalous pulmonary venous connections. J Comput Assist Tomogr 1994; 18:243.
  15. Shen Q, Pa M, Hu X, Wang J. Role of plain radiography and CT angiography in the evaluation of obstructed total anomalous pulmonary venous connection. Pediatr Radiol 2013; 43:827.
  16. BURROUGHS JT, EDWARDS JE. Total anomalous pulmonary venous connection. Am Heart J 1960; 59:913.
  17. Ward KE, Mullins CE. Anomalous pulmonary venous connections, pulmonary vein stenosis, and atresia of the common pulmonary vein. In: The Science and Practice of Pediatric Cardiology, Garson A Jr, Bricker JT, Fisher DJ, Neish SR (Eds), Williams and Wilkins, Baltimore 1998. p.1431.
  18. Ishino K, Alexi-Meskishvili V, Hetzer R. Preoperative extracorporeal membrane oxygenation in newborns with total anomalous pulmonary venous connection. Cardiovasc Surg 1999; 7:473.
  19. Raisher BD, Grant JW, Martin TC, et al. Complete repair of total anomalous pulmonary venous connection in infancy. J Thorac Cardiovasc Surg 1992; 104:443.
  20. Lock JE, Bass JL, Castaneda-Zuniga W, et al. Dilation angioplasty of congenital or operative narrowings of venous channels. Circulation 1984; 70:457.
  21. Meadows J, Marshall AC, Lock JE, et al. A hybrid approach to stabilization and repair of obstructed total anomalous pulmonary venous connection in a critically ill newborn infant. J Thorac Cardiovasc Surg 2006; 131:e1.
  22. Hancock Friesen CL, Zurakowski D, Thiagarajan RR, et al. Total anomalous pulmonary venous connection: an analysis of current management strategies in a single institution. Ann Thorac Surg 2005; 79:596.
  23. Sinzobahamvya N, Arenz C, Brecher AM, et al. Early and long-term results for correction of total anomalous pulmonary venous drainage (TAPVD) in neonates and infants. Eur J Cardiothorac Surg 1996; 10:433.
  24. Bando K, Turrentine MW, Ensing GJ, et al. Surgical management of total anomalous pulmonary venous connection. Thirty-year trends. Circulation 1996; 94:II12.
  25. Michielon G, Di Donato RM, Pasquini L, et al. Total anomalous pulmonary venous connection: long-term appraisal with evolving technical solutions. Eur J Cardiothorac Surg 2002; 22:184.
  26. Boger AJ, Baak R, Lee PC, et al. Early results and long-term follow-up after corrective surgery for total anomalous pulmonary venous return. Eur J Cardiothorac Surg 1999; 16:296.
  27. Caldarone CA, Najm HK, Kadletz M, et al. Surgical management of total anomalous pulmonary venous drainage: impact of coexisting cardiac anomalies. Ann Thorac Surg 1998; 66:1521.
  28. Jenkins KJ, Sanders SP, Orav EJ, et al. Individual pulmonary vein size and survival in infants with totally anomalous pulmonary venous connection. J Am Coll Cardiol 1993; 22:201.
  29. Frommelt PC, Sheridan DC, Deatsman S, et al. Unobstructive total anomalous pulmonary venous return: impact of early elective repair on the need for prolonged mechanical ventilatory support. Pediatr Cardiol 2010; 31:1191.
  30. St Louis JD, Harvey BA, Menk JS, et al. Repair of "simple" total anomalous pulmonary venous connection: a review from the Pediatric Cardiac Care Consortium. Ann Thorac Surg 2012; 94:133.
  31. Duff JP, Joffe AR, Vatanpour S, et al. Neurocognitive outcomes at kindergarten entry after surgical repair of total anomalous pulmonary venous connection in early infancy. Pediatr Cardiol 2015; 36:350.
  32. Hyde JA, Stümper O, Barth MJ, et al. Total anomalous pulmonary venous connection: outcome of surgical correction and management of recurrent venous obstruction. Eur J Cardiothorac Surg 1999; 15:735.
  33. Marino BS, Lipkin PH, Newburger JW, et al. Neurodevelopmental outcomes in children with congenital heart disease: evaluation and management: a scientific statement from the American Heart Association. Circulation 2012; 126:1143.
  34. Saxena A, Fong LV, Lamb RK, et al. Cardiac arrhythmias after surgical correction of total anomalous pulmonary venous connection: late follow-up. Pediatr Cardiol 1991; 12:89.
  35. Tanel RE, Kirshbom PM, Paridon SM, et al. Long-term noninvasive arrhythmia assessment after total anomalous pulmonary venous connection repair. Am Heart J 2007; 153:267.